Electrical Status Epilepticus During Sleep Research Articles

Using scalp EEG to predict seizure recurrence and electrical status epilepticus in children with idiopathic focal epilepsy

PurposeSome individuals with idiopathic focal epilepsy (IFE) experience recurring seizures accompanied by the evolution of electrical status epilepticus during sleep (ESES). Here, we aimed to develop a predictor for the early detection of seizure recurrence with ESES in children with IFE using resting state electroencephalogram (EEG) data. MethodsThe study group included 15 IFE patients who developed seizure recurrence with ESES. There were 17 children in the control group who did not experience seizure recurrence with ESES during at least 2-year follow-up. We used the degree value of the partial directed coherence (PDC) from the EEG data to predict seizure recurrence with ESES via 6 machine learning (ML) algorithms. ResultsAmong the models, the Xgboost Classifier (XGBC) model achieved the highest specificity of 0.90, and a remarkable sensitivity and accuracy of 0.80 and 0.85, respectively. The CATC showed balanced performance with a specificity of 0.85, sensitivity of 0.73, and an accuracy of 0.80, with an AUC equal to 0.78. For both of these models, F4, Fz and T4 were the overlaps of the top 4 features. ConclusionsConsidering its high classification accuracy, the XGBC model is an effective and quantitative tool for predicting seizure recurrence with ESES evolution in IFE patients. We developed an ML-based tool for predicting the development of IFE using resting state EEG data. This could facilitate the diagnosis and treatment of patients with IFE.

An attention-based deep learning method for the detection of electrical status epilepticus during sleep from electroencephalogram waveform analysis in children

Electrical status epilepticus during sleep (ESES) is a special interictal electroencephalographic phenomenon observed in children with epilepsy. ESES refers to continuous or nearly continuous epileptiform discharges of bilateral (or unilateral) spikes and waves induced during non-rapid eye movement (NREM) sleep. It mainly manifests in a series of childhood epileptic syndromes characterized by seizures, ESES, and cognitive impairment, which seriously affect the health of children. Typically, the spike-wave index (SWI), which is the percentage of spikes and waves duration in the total NREM time, is an important criterion for diagnosing ESES. The SWI measures the proportion of the total duration of spikes and waves to the NREM sleep time within several hours. Currently, SWI is obtained primarily by manual measurement and calculation, which has a certain subjective bias, low accuracy, time and labor consumption, and is a waste of medical resources. Therefore, we designed a novel data segmentation model based on a multiresolution convolutional neural network and a self-attention mechanism to calculate the SWI quickly and accurately. This can assist clinicians in making more accurate judgments and providing a better guide for the treatment of children with epilepsy. A large amount of clinical patient data was used to test the proposed model. The results confirmed that this model has an acceptable generalization ability for data from different patients and the same patient at different times. Each performance index of the model was superior to that of current common models, thus fulfilling the requirements for clinical testing.

The prospective study of 54 children with ESES: how to simplify the EEG diagnosis and guide the treatment.

To simplify the electroencephalogram (EEG) diagnosis and guide the treatment of electrical status epilepticus during sleep (ESES). We recruited 54 children with ESES from December 2019 to December 2020 and compared various spike-wave index (SWI) calculation methods. Time-frequency analysis assessed the correlation between high-frequency oscillations (HFOs) energy and the SWI. We divided 42 children into responder and non-responder treatment groups based on the observations made during a 12-month follow-up period and evaluate different treatment and the independent risk factors of refractory ESES. The SWI of five minutes before the second sleep cycle of non-rapid eye movement (NREM; long method II) and that of all NREM sleep (total method) were not significantly different (P = 0.06). The average energy of γ (r = 0.288, P = 0.002) and ripple (r = 0.203, P = 0.04) oscillations were correlated with the SWI. Encephalomalacia was an independent risk factor for refractory ESES (OR: 10.48, 95% CI: 1.62-67.63). The clinical seizure improvement rates of ASMs, ASMs with benzodiazepines, and ASMs with benzodiazepines and steroids after 12 months were 9.3%, 42.8%, and 53.8%, EEG improvement rate were 5.5%, 30.9% and 37%, respectively. The intelligence of the children in the responder treatment group has improved during the 1-year follow-up. These findings demonstrate EEG and clinical features of ESES and may provide basis for simplifying diagnosis and guiding the treatment of children with ESES.

Role of various antiepileptic drugs in electrical status epilepticus during sleep and its complications

Electrical Status Epilepticus during Sleep (ESES) is an uncommon epileptic encephalopathy typically seen in children. It features pronounced epileptiform activity during non-REM sleep stages and is linked with behavioral and cognitive impairments. This article introduces a novel method for managing patients with ESES.

Ocular emotion discrimination disorders in self-limited epilepsy patients with centrotemporal spikes complicated with electrical status epilepticus during sleep: A pediatric neuropsychological study.

We investigated the characteristics and factors influencing eye emotion recognition in self-limited epilepsy patients with centrotemporal spikes (SeLECTS) complicated with electrical status epilepticus during sleep (ESES). We selected SeLECTS (n=160) patients treated in the outpatient and inpatient departments of Anhui Children's Hospital from September 2020 to January 2022. According to the video electroencephalogram monitoring slow-wave index (SWI), SeLECTS patients with SWI<50% were assigned into the typical SeLECTS group (n=79), and patients with SWI ≥ 50% were assigned into the ESES group (n=81). Patients in the two groups were assessed by The Eye Basic Emotion Discrimination Task (EBEDT) and The Eye Complex Emotion Discrimination Task (ECEDT), respectively. Comparisons were made with age-, sex- and education level-matched healthy control participants. The correlation between the characteristics of emotional discrimination disorder in the eye area and the clinical influencing factors was analyzed in ESES group, and p ≤ .050 was the threshold for significance. Relative to the healthy control group, scores of sadness and fear in the typical SeLECTS group were markedly lower (p=.018, p=.023), while differences in scores of disgust, happiness, surprise, and anger were not significantly different between the groups (p=.072, p=.162, p=.395, p=.380, respectively). Compared with the healthy control group, the ESES group had significantly low scores in recognition of sadness, fear, disgust, and surprise (p=.006, p=.016, p=.043 and p=.038, respectively). However, differences in recognition of happiness and anger between the groups were not significant (p=.665 and p=.272). Univariate logistic analysis showed that the score of eye recognition for sadness in the ESES group was affected by age of onset, SWI, ESES duration and number of seizures. The score of eye recognition for fear was mainly affected by SWI, while the score of eye recognition for disgust was affected by SWI and number of seizures. The surprised eye emotion recognition score was mainly affected by the number of seizures. Variables with p<.1 were considered to be independent variables of multivariable ordered logistic regression. Multivariate logistic analysis showed that sadness emotion recognition was mainly affected by SWI and ESES duration, while disgust was mainly affected by SWI. The typical SeLECTS group showed impaired emotion (sadness and fear) recognition function in the eye area. The ESES group was associated with more intense emotional (sadness, fear, disgust, and surprise) recognition impairment in the eye region. The higher the SWI, the younger the onset age and the longer the duration of ESES, while the more the number of seizures, the more serious the impairment of emotional recognition function in the affected eye area.

Electrical Status Epilepticus during Sleep in a Male Filipino with Rare Nonsense Mutation Variant of Sotos Syndrome on Carbamazepine Monotherapy

Electrical status epilepticus during sleep (ESES) is an electrographic pattern associated with specific genetic disorders, brain malformations, and use of some antiseizure medications. This case report aims to present the management of ESES in Sotos syndrome (SoS) on carbamazepine.A nine-year-old Filipino male with clinical features suggestive of overgrowth syndrome presented with febrile seizure at one year old. Cranial imaging showed cavum septum pellucidum, corpus callosal dysgenesis, and ventriculomegaly. He was on carbamazepine monotherapy starting at three years old. A near continuous diffuse spike–wave discharges in slow wave sleep was recorded at nine years old hence shifted to valproic acid. Follow-up study showed focal epileptiform discharges during sleep with disappearance of ESES. Next generation sequencing tested positive for rare nonsense mutation of nuclear receptor binding set-domain protein 1 confirming the diagnosis of SoS.Advanced molecular genetics contributed to determination of ESES etiologies. To date, this is the first documented case of SoS developing ESES. Whether an inherent genetic predisposition or drug-induced, we recommend the avoidance of carbamazepine and use of valproic acid as first-line therapy. &nbsp; &nbsp

Microstructural evaluation of the brain with advanced magnetic resonance imaging techniques in cases of electrical status epilepticus during sleep (ESES)

Microstructural evaluation of the brain with advanced magnetic resonance imaging techniques in cases of electrical status epilepticus during sleep (ESES)

Effectiveness of perampanel in the treatment of pediatric patients with focal epilepsy and ESES: A single-center retrospective study.

Objective: To investigate the therapeutic effect and influencing factors of perampanel (PER) on electrical status epilepticus during sleep (ESES). Methods: We retrospectively analyzed the clinical data of pediatric patients with focal epilepsy and ESES who were treated at the Epilepsy Center of Shengjing Hospital of China Medical University between January 2016 and March 2022. Changes in the spike wave index (SWI) after 24 weeks of PER add-on treatment were compared. Kaplan‒Meier survival analysis, the log-rank test and multivariate Cox regression analysis were performed. Results: A total of 54 pediatric patients met the inclusion criteria, including 33 males and 21 females. The mean age at the diagnosis of epilepsy was 6.41 ± 2.14 years and at ESES diagnosis was 7.58 ± 2.40 years. The mean ESES duration before add-on PER was 25.31 ± 15.12 months. The mean age of the patients at add-on PER initiation was 9.69 ± 2.12 years. The ESES resolved in 29 children after 6 months of PER add-on treatment, and the response rate was 53.7%. Univariate analysis with the log-rank test showed that the therapeutic effect of PER differed according to the age at ESES diagnosis and ESES duration before add-on PER treatment. Multivariate Cox regression analysis showed that only ESES duration before PER administration was a risk factor for PER treatment failure, and the other factors had no effect on the therapeutic effect. Conclusion: PER add-on treatment has a good therapeutic effect on ESES and can be used as an alternative to corticosteroid and benzodiazepines. The therapeutic effect of PER add-on treatment was not related to the dose. A longer ESES duration results in a worse therapeutic effect. Therefore, more aggressive treatment measures should be implemented for ESES.

Efficacy and adverse reactions of perampanel in the treatment of epilepsy in children.

ObjectiveTo observe the clinical effect and adverse reactions of perampanel in the treatment of epilepsy in children.MethodsA retrospective analysis was performed on 83 children with epilepsy who were treated with perampanel in the Department of Pediatric Neurology, Second Affiliated Hospital of Xi'an Jiaotong University from April to August 2021. The treatment status, prognosis and adverse reactions were followed up. The effective rates of different age groups, different seizure types and epilepsy syndromes, and different treatment methods were statistically analyzed. The effective rate and adverse reactions of all patients were statistically analyzed.ResultsThe overall effective rate of perampanel in the treatment of epilepsy was 62.03%, and there was no significant difference in the effective rate of perampanel in the treatment of epilepsy in patients of different ages (P > 0.05). The effective rates of perampanel in the treatment of focal seizures and generalized seizures were 60.38% and 65.38%, and the effective rates of benign childhood epilepsy with centrotemporal spikes (BECT), BECT combined with electrical status epilepticus during sleep (ESES) and frontal lobe epilepsy (FLE) were 88.89, 72.73, and 66.67%. The effective rates of monotherapy and combination therapy were 88.88 and 58.57%, respectively. The above statistical differences were not statistically significant (P > 0.05). In addition, the adverse reaction rate of perampanel treatment was 16.45%, including irritability, drowsiness, dizziness, nausea, vomiting and abnormal liver function.ConclusionPerampanel has a high efficiency and controllable adverse reactions in the treatment of childhood epilepsy. This drug can be used as a reliable choice for long-term use in the treatment of epilepsy in children.

Continuous Spikes and Waves During Sleep (CSWS), Severe Epileptic Encephalopathy, and Choreoathetosis due to Mutations in FRRS1L.

Background. Biallelic pathogenic variants in the FRRS1L gene are now known to cause developmental and epileptic encephalopathy-37 (DEE37). It can also be associated with chorea and continuous spikes and waves during sleep (CSWS). CSWS is a rare age-related epileptic encephalopathy syndrome of childhood that is characterized by seizures, neurocognitive regression and electrical status epilepticus during sleep (ESES) on electroencephalogram (EEG) that evolves in four stages. Seizures start during the prodromal phase but the ESES on EEG appears only during acute stage and this is the stage when the diagnosis of CSWS can be made. Methods. We present two patients with FRRS1L mutation causing DEE37 with CSWS. We also review twenty-nine cases of DEE37 described in the literature before and discuss its association with CSWS in the total cohort of thirty-one cases. Results. Developmental regression was found in 80% of the patients, mean age of seizure onset was 18 months, ESES or slow spike and wave on the EEG were reported mostly in the older patients (median age of 11 years) and hypsarrhythmia was reported in younger patients (median age of 4 years). This could suggest that if the younger patients were followed longer their EEG would have evolved into ESES during the acute stage of this syndrome and a diagnosis of CSWS could be made. Conclusion. Recognizing ESES and the natural evolution of CSWS is important in diagnosis and proper management of these patients. More detailed report of EEG findings and the evolution of epilepsy and development are needed to further characterize this syndrome.

Hemimegalencephaly and Epileptic Encephalopathy Associated with a Variant of Uncertain Significance of the TRIO Gene

Objectives: Electrical status epilepticus during sleep (ESES) is a type of EEG pattern seen in children with childhood-onset epileptic seizures and cognitive, language and motor regression. ESES has been associated with different etiologies, with or without structural abnormalities of the brain. To replace the term “ESES”, the recent Position Paper of the ILAE Taskforce on Nosology and Definitions introduced the term Epileptic Encephalopathy with Spike-Wave Activity during Sleep (EE-SWAS), which represents an activation of epileptiform activity, typically 1.5-2.5 Hz during NREM sleep, in a previously normal child and leads to cognitive and motor regression. Pathogenic variants in the TRIO gene are associated with autosomal dominant mental retardation type 44 (MRD44), which is characterized by mildly delayed global development resulting in variable intellectual deficits, learning difficulties, and variable dysmorphic features mostly represented by facial asymmetry, microcephaly, abnormalities of the fingers, and dental anomalies. Materials and methods: We present the case of a 13-year-old girl with onset of atonic seizures at the age of 3 who had undergone different types of antiepileptic drug (AED) therapies and was seizure-free 4 years later. She had an acquired impaired cognitive status as well as hemimegalencephaly, multiple hypopigmented patches on her legs and a large patch of hypopigmentation on the left side of her face extending towards the scalp (Becker’s nevus), dental anomalies (gingival hyperplasia, ogival palate), periventricular gliosis and electroencephalographic and (EEG) findings consistent with SWAS. Outcomes: Genetic testing confirmed a heterozygous variant of uncertain significance (VUS) of the TRIO gene, possibly associated with autosomal dominant MRD44. Although our patient presented with some phenotypic similarities to MRD44, her cognitive impairment improved with control of the SWAS pattern and AED adjustments. Conclusion: Association of structural anomalies of white matter, hemimegalencephaly, and encephalopathy with SWAS and a VUS mutation of the TRIO gene might suggest a different genetic neurocutaneous syndrome altogether.

Electrical status epilepticus during sleep electroencephalogram waveform identification and analysis based on a graph convolutional neural network

Electrical status epilepticus during sleep (ESES) is an epileptic syndrome in which neurons in the brain continue to discharge during the sleep phase and is common in mid-childhood. Affected patients often experience a decline in cognition, learning ability, memory, and expressive language skills. Therefore, timely and accurate diagnosis can effectively protect the health of a patient. Currently, the identification and analysis of ESES activities mainly rely on manual detection or traditional matching learning algorithms, such as morphology and template matching. These algorithms are time-consuming or have low accuracy. Therefore, in this paper, we propose a graph convolutional neural network that can automatically and accurately identify ESES activity from non-ESES activity. We divide the whole EEG signal into small segments, each of which covers one second of the EEG data. Then, we construct a graph according to each segment of the EEG data and train a graph convolutional neural network to classify the graph into two categories: ESES or non-ESES. Compared with other state-of-the-art algorithms, for the proposed algorithm, the accuracy, F1-Score, Area Under Curve(AUC) and sensitivity reaches 91.2%, 95.0%, 96.5%, and 91.3%, respectively, and outperforms the other algorithms.

Electrical status epilepticus during sleep (ESES) in benign childhood epilepsy with Centrotemporal spikes (BCECTS): insights into predictive factors, and clinical and EEG outcomes.

Benign childhood epilepsy with centro-temporal spikes (BCECTS), otherwise known as benign rolandic epilepsy, is the most common focal epilepsy in childhood. This study aimed to evaluate the development and resolution of ESES in children with BCECTS and evaluate the clinical and electroencephalography (EEG) parameters associated with prognosis. Resolution of ESES was defined as the reduction of the spike-wave index (SWI) to <50%. The SWI short method, measurements from the first 180 s of non-rapid eye movement; and the conventional method, measurements from total NREM stage 2, SW count during the first 60 and 180 s of NREM, SW localization, and ESES type were determined. Of a total of 126 BCECTS patients, 33, including 13 females, 20 males, who developed ESES during follow-up, were included in the study. ESES remission was observed in 42.4% (n = 14) of the patients. The median time to remission was 10.5 months. The rate of resolution was 87.9 % for the entire population. The mean age at resolution was 9.8 ± 2.05 years and the mean time to resolution was 8.8 months. The data demonstrated that age at ESES diagnosis, the time between BCECTS diagnosis and the onset of ESES, time to resolution of ESES, ESES remission, and seizure freedom after ESES were significantly associated with prognosis. The early recognition of ESES evolution in children with BECTS, the better understanding of the relationship between age at ESES diagnosis and remission and prognosis, and timely intervention can prevent long-term sequelae.

On the role of REM sleep microstructure in suppressing interictal spikes in Electrical Status Epilepticus during Sleep

ObjectiveNon-Rapid Eye Movement (NREM) sleep promotes the spread and propagation of Interictal Epileptiform Discharges (IEDs), while IEDs are suppressed during REM. Recently, it has been shown that the inhibitory effect on epileptic activity is mostly exerted by the phasic REM (PREM) microstate. This study aims at assessing if this holds true even in the extreme condition of IEDs activation during sleep represented by Electrical Status Epilepticus during Sleep (ESES). Methodseight patients affected by ESES, who underwent long-term EEG were included. REM was subdivided into phasic and tonic microstates along with the sleep scoring. IEDs count was carried out using a semi-automatic method and a Spike Index (SI) was calculated. ResultsThe SI was significantly higher in NREM sleep than in REM. Within REM, the SI was significantly lower in PREM than in tonic REM (TREM). The SI was reduced by 84% in TREM with respect to NREM and by 97% in PREM with respect to NREM. Moreover, the SI was reduced by 87% in PREM with respect to TREM. ConclusionsPREM has a greater suppressive effect on epileptic activity even in the extreme IEDs activation during sleep typical of ESES. SignificanceUnderstanding the protective effect of PREM sleep on epileptic activity might be relevant for future therapeutic approaches.

A case of Landau-Kleffner syndrome with SLC26A4-related hearing impairment

BackgroundLandau-Kleffner syndrome (LKS) is an acquired aphasia and electroencephalogram (EEG) abnormalities mainly in temporoparietal areas. SLC26A4 mutations can cause hearing loss associated with enlarged vestibular aqueduct (EVA).Case presentationsWe report a case of LKS in a 5-year-old boy with non-syndromic EVA due to homozygous mutations of c.919-2A>G (IVS7-2A>G) in SLC26A4. He had normal language development before 2 years old. At the age of 2.5 years, he was admitted to the hospital due to remarkable language delay, and diagnosed with hearing loss with EVA. The seizures started at 4.4 years of age and EEG recording showed electrical status epilepticus during sleep (ESES) with a posterior-temporal predominance. He received cochlear implantation in the right ear at 4.7 years of age, which improved his hearing and language skills. The nocturnal focal motor seizures recurred at 4.9 years of age. Then a remarkable inability to respond to calls and reduction in spontaneous speech were noticed. He was treated with methylprednisolone at 5 years old, which controlled the seizures, suppressed ESES, and remarkably improved the language ability. The absence of seizures maintained until the last follow-up at 5.3 years of age, with further improvements in EEG recording and language ability.ConclusionsThe co-existence of LKS and hearing loss caused by SLC26A4 mutations increases the difficulty of LKS diagnosis, especially in the presence of hearing loss and impaired language skills. EEG discharges predominantly in temporoparietal areas, the occurrence of ESES, and language improvement after antiepileptic medications are potential indicators for LKS diagnosis.

A Hybrid Expert System for Individualized Quantification of Electrical Status Epilepticus During Sleep Using Biogeography-Based Optimization

Electrical status epilepticus during sleep (ESES) is an epileptic encephalopathy in children with complex clinical manifestations. It is accompanied by specific electroencephalography (EEG) patterns of continuous spike and slow-waves. Quantifying such EEG patterns is critical to the diagnosis of ESES. While most of the existing automatic ESES quantification systems ignore the morphological variations of the signal as well as the individual variability among subjects. To address these issues, this paper presents a hybrid expert system that dedicates to mimicking the decision-making process of clinicians in ESES quantification by taking the morphological variations, individual variability, and medical knowledge into consideration. The proposed hybrid system not only offers a general scheme that could propel a semi-auto morphology analysis-based expert decision model to a fully automated ESES quantification with biogeography-based optimization (BBO), but also proposes a more precise individualized quantification system to involve the personalized characteristics by adopting an individualized parameters-selection framework. The feasibility and reliability of the proposed method are evaluated on a clinical dataset collected from twenty subjects at Children's Hospital of Fudan University, Shanghai, China. The estimation error for the individualized quantitative descriptor ESES is 0-4.32% and the average estimation error is 0.95% for all subjects. Experimental results show the presented system outperforms existing works and the individualized system significantly improves the performance of ESES quantification. The favorable results indicate that the proposed hybrid expert system for automatic ESES quantification is promising to support the diagnosis of ESES.

Genetic Etiologies in Developmental and/or Epileptic Encephalopathy With Electrical Status Epilepticus During Sleep: Cohort Study.

BackgroundRecently, the electroencephalogram pattern of electrical status epilepticus during sleep (ESES) had been reported in some genetic disorders, and most of them were noted with developmental and epileptic encephalopathy (DEE) or epileptic encephalopathy (EE). This study aimed to determine the genetic etiologies and clinical characteristics of ESES in DEE/EE.MethodsWe performed a cohort study in cases of DEE or EE with ESES. Tio-based genetic testing was performed in 74 cases and was analyzed to identify underlying variants.ResultsPathogenic or likely pathogenic variants were identified in 17/74 cases, including KCNQ2 (n = 6), KCNA2 (n = 5), GRIN2A (n = 3), SLC9A6 (n = 1), HIVEP2 (n = 1), and RARS2 (n = 1). Eleven were boys. The median age at seizure onset was 6 months. ESES occurred at the mean age of 2.0 ± 1.2 years, predominant in the Rolandic region in 14 years. Twelve of 17 cases had the first stage of different epilepsy preceding ESES: 2/12 were diagnosed as Ohtahara syndrome, 2/12 were diagnosed as infantile spasms, 3/12 were diagnosed as DEE, and 5/12 were diagnosed as EE without the epileptic syndrome.ConclusionMonogenic variants explained over 20% of DEE/EE with ESES. ESES could be an age-related feature in genetic disorders and occurred after the first stage of different epilepsy. Both age-related factors and genetic etiology were suggested to play a role in the occurrence of ESES in genetic DEE/EE.

A knowledge-based approach for automatic quantification of epileptiform activity in children with electrical status epilepticus during sleep

Objective. Electrical status epilepticus during sleep (ESES), as electroencephalographic disturbances, is characterized by strong activation of epileptiform activity in the electroencephalogram during sleep. Quantitative descriptors of such epileptiform activity can support the diagnose and the prognosis of children with ESES. To quantify the epileptiform activity of ESES, a knowledge-based approach to mimic the clinical decision-making process is proposed. Approach. Firstly, a morphological operations-based scheme is designed to quickly locate the positive peaks/negative pits and roughly estimate the onset/offset of spike and slow-wave abnormalities. Then, to provide the accurate duration of ESES patterns, a set of rules for further adjusting these onsets/offsets are proposed by merging medical knowledge with a generalized threshold obtained from statistics. As such, the quantification is accomplished by evaluating the obtained spike and slow-wave abnormalities and their various durations. Main results. The effectiveness and feasibility of the proposed method were evaluated on a clinical dataset that collected at Children’s Hospital of Fudan University, Shanghai, China. We demonstrate that the proposed method can recognize different types of spike and slow-wave abnormalities. The sensitivity, precision, and false positive rate achieved 91.96%, 97.09%, and 1.88 min−1, respectively. The estimation error for the spike-wave index was 2.32%. Comparison results showed that our method outperforms the state-of-the-art. Significance. The quantification of spike and slow-waves provides information about ESES activity. The detection of variations types of spike and slow-waves improves the performance in the quantification of ESES. Experimental results suggest that the proposed method has great potential in automatic ESES quantification and can help improve the diagnosis and researches of epileptic encephalopathy with ESES.

Neuropsychological Deficits in Patients with Electrical Status Epilepticus During Sleep: A Non-invasive Analysis of Neurovascular Coupling.

To evaluate the effects of electrical status epilepticus during sleep (ESES) on cerebral blood flow (CBF) and explore the associated neuro-vascular coupling and neuropsychological deficits. 19 ESES patients were recruited to undergo real-time transcranial doppler ultrasonography (TCD) and video-EEG monitoring (vEEG). Patients were grouped based on their cognitive functions or their EEG patterns. The mean cerebral blood flow velocity (CBFVm) of the unilateral middle cerebral artery was measured using TCD and was used to calculate various relevant parameters. The 19 patients participated in a total of 54 effective TCD-vEEG monitoring sessions. We found a significant effect of clinical severity for the following measurements: spike wave index (SWI), peak and average deep sleep stage (N3) CBFVm, peak, average and minimum deep sleep and awake CBFVm, and CBFVm oscillations during deep sleep. Nevertheless, CBFVm oscillations were not related to SWI. Furthermore, CBFVm oscillations revealed a statistically significant difference between the near-ESES and asymmetric-ESES groups. CBFVm oscillations may reflect the neuro-vascular coupling process associated with ESES disfunction. Understanding the relationship between CBFVm oscillations and epileptic activity will be important for assessing the neuropsychological damage associated with ESES and for developing treatment options for this and other diseases.

Effect of methylprednisolone shock therapy on immune function of electrical status epilepticus during sleep in children

Objective To study the effect of methylprednisolone shock therapy on electrical status epilepticus during sleep (ESES) in children and the changes of immune function before and after methylprednisolone shock therapy. Methods Thirty-five children hospitalized in pediatric neurology ward of Shengjing Hospital of China Medical University from October 2017 to October 2018 were selected, including 19 children with newly diagnosed temporal lobe epilepsy (TLE group) and 16 children with initial diagnosis of TLE with ESES (ESES group), and 21 healthy children in the same period were selected as normal control group.A total of 32 cases with ESES who were taken oral administration of more than 2 anti-epileptic drugs(AEDs)ineffective were given methylprednisolone shock treatment for 3 days.Changes of lymphocyte subsets and cytokines among groups were compared. Results The longer the course of disease, the worse the prognosis of children with ESES.Compared with normal control group, the NK cell activity were significantly lower in children of ESES group(P<0.05). The levels of IL-6 were higher in children of ESES group compared with that in children of TLE group, and there was significant difference between two groups(P<0.05). In children with ESES who had poor therapeutic effect of oral AEDs, the proportion of peripheral blood B lymphocytes increased after methylprednisolone shock treatment, and the proportion of T lymphocyte subsets and NK cells decreased, and the differences were statistically significant(P<0.05). The levels of IL-2, IL-4 and IL-10 were higher than those before treatment, the levels of IL-6, IL-17, INF-γ and TNF were lower than those before treatment, and the difference between the level of IL-6 before and after treatment was statistically significant(P<0.05). EEG spike waves index decreased significantly after treatment, some children′s growth and development and cognitive function improved, all children had no serious infection, high blood pressure, electrolyte imbalance and other adverse reactions.The correlation between therapeutic effect and changes of immune indexes was statistically significant in levels of NK cells(r=0.50, P<0.01), B lymphocytes (r=0.35, P=0.04) and IL-6(r=0.46, P=0.01), and all of them were positively correlated(P<0.05). Conclusion Children with ESES have immune dysfunction and may have excessive inactivation of NK cells and elevated levels of IL-6.Congenital immunity and adaptive immunity may play a role in the pathogenesis and pathophysiology of ESES syndrome.Methylprednisolone shock therapy could significantly reduce the non-rapid eye movement discharge index in children with ESES.The mechanism may be related to the significant decrease of IL-6 level.It may also be related to redistribution of lymphocyte subsets, affecting cell differentiation and balancing various cytokines. Key words: Electrical status epilepticus during sleep; Lymphocyte; Cytokines; Methylprednisolone